Q&A – My Aniridia & Nystagmus

A few of my recent posts about my visual impairment have become very popular, which has been wonderful of course. In large part this is because the RNIB have very kindly shared them, which has enabled them to reach a huge audience.

As a result of those posts, various people have got in touch with questions relating to my conditions. I’ve answered them all either privately or in the comments area they were posted in. But I thought I’d summarise the questions and my responses here as well, in case others find it useful.

Thank you to Sam, Charlotte and Nadine for the questions in this post, I’m happy to help as best I can. And if any of you reading this want to ask me anything about my visual impairment, or anything else for that matter, then feel free to comment here or on my Twitter or Facebook feeds. I’m happy to do more Q&A posts like this if people wish.

So here are the questions and answers. I hope you find it interesting and useful. I’ve also made a video of my responses as well.

Sam

What do you think affects your vision more? Aniridia or nystagmus? For example, you might believe aniridia is 35% of the reason for worse sight and nystagmus is 65% of the reason?

Having never had either condition in isolation, it’s hard to quantify the effect they each have. There is a level of crossover, and one is responsible for the other (nystagmus came as part of the package deal with aniridia, as it does for other conditions too – doesn’t for everyone, but the odds of it are high in my case).

I would say nystagmus affects me more though, as I always notice its effects. I always have dodgy distance vision, I always need things close up to read them. Aniridia is more about sensitivity to light and difficulty to see in the dark, so when I’m indoors under general lighting conditions, I’m often ok. And I can reduce the glare from computer screens by inverting the colours. But I still notice the effects of nystagmus even when the lighting’s right (I don’t notice the shaking unless my eyes are really tired, it’s just the effects it has on my focusing that I notice most).

So when I’m indoors in comfortable lighting, nystagmus is the most dominant, so could be like 75% of the reason for my poor sight. When I’m out and about though, the aniridia affects me much more, so it’s much more 50/50 between the two, possibly even 80/20 in favour of aniridia if it’s particularly glary or dark.

How do you think your vision would be if you just had nystagmus? Do you think your eye movement has slowed/changed since you were a baby?

If I just had nystagmus, my distance vision would still be bad. The absence of aniridia probably wouldn’t change that – as I say, under the best lighting conditions, I still can’t focus on things at a distance. If I just had aniridia instead, though, then maybe my distance vision would be a bit better than it is. Although even that’s not guaranteed. Aniridia still has its own effects on distance vision – even though light sensitivity is the most obvious outcome, it can have other effects as well, depending on what variant and severity of aniridia you have.

My nystagmus isn’t going to get better, but it might not get any worse either. My sight in general is likely to get worse over time though, but due to other effects. I’m more likely to develop glaucoma and cataracts for instance. So the only thing I can do is attempt to stabilise it if I notice any deterioration (e.g. if I develop cataracts, they could be removed). I haven’t noticed anything getting worse yet though – as far as I can tell, my sight has been stable so far. Of course, it could be that any deterioration has been so slow and gradual that I haven’t noticed. That wouldn’t surprise me ro be honest.

What do you think about the speed of eye movement? In other words, if you have rapid side to side eye movement in short distances, do you think that is better or worse/harder for seeing compared to one with slower side movement that has longer travel?

Without experiencing both the slow and rapid variations, it’s hard to say how they compare. Logic would suggest that the more rapid the eye movements, the harder it is to focus on things, because your eyes have less time to pick up any information, and your brain will thus find it a lot harder to process that information and adapt to it. My eye movements are relatively slow and short I think, so my brain has adapted quite well. For other people that might not be the case, but as I’m not in their shoes I simply don’t know.

How is your depth perception?

My depth perception isn’t great. I can see the world around me, but anything at a distance is blurred, increasingly so the further away it gets. If I want to read things or see fine detail, they need to be close to me, or very large if they’re at a distance, or I’ll use my monocular (a little telescope) to see things. In terms of telling you how far something is in metres or whatever, I’m hopeless at that. If I’m jumping off something, judging the distance to land properly can also be difficult. And as for staircases, if the steps are well-defined, with clear edges and in good lighting, then I can go down them without any difficulty. But if the edges aren’t marked and they’re all the same colour, then I will have to take my time to figure out how deep they are.

How is your speed of tracking?

My speed of tracking is poor. If someone throws something at me to catch, then I’m very unlikely to catch it, as I can’t figure out where it is quickly enough to be able to react quickly enough. Or if a bike is zooming down the pavement, the first I’ll know is when it whizzes right by me, not before. If some text comes up on a TV show for just a few seconds, then I might not be able to read it fast enogh. And so on. In other words, I’m better when things are slower. I generally need a few seconds longer to process what I’m seeing compared to other people.

Have you met others with nystagmus?

I’ve met and am friends with lots of people who have it, yes. I went to a school for the visually impaired, so grew up knowing other children with a whole spectrum of conditions, including nystagmus in quite a few cases. And I’ve got to know more people with nystagmus since moving to London, as a result of my blogging and hooking up with VI social groups. Plus I attended (and spoke at) the Nystagmus Network’s Open Day a month ago, where everyone has it. There’s a lot of us out there!

Are there actually folks you know with nystagmus who have pretty good visual acuity e.g. can see road signs, bikes, balls coming to them, etc?

The effects of nystagmus do vary, so there have been people who are a bit better at things than me, sure, either because their nystagmus isn’t quite as bad, and/or they don’t have the additional condition of aniridia that I have, which complicates things in my case. So now and again I’ll be with someone who can make out things like signs a bit easier than me, and as a kid one or two of my friends were better at some video games than me because their reactions were quicker. I don’t personally know anyone with nystagmus who can drive, but if it’s minor enough then it’s potentially possible. It wouldn’t surprise me if there were a few drivers with nystagmus out there.

Charlotte

I have spoken to a few families with aniridia and nystagmus but none with both. My daughter’s aniridia isn’t full aniridia apparently, which confuses a lot of people. They call it iris hypoplasia or a variation / form of aniridia. Have you ever been familiar with this?

Everyone with aniridia sees things in a different way depending on how much of the iris they’ve lost or how damaged it is, and what other conditions they may have on the side, So it’s perfectly possible for someone not to have ‘full’ aniridia

I had to look up the term, not being a doctor, but according to Wikipedia, hypoplasia is “underdevelopment or incomplete development of a tissue or organ”, which would make sense here. It tallies with what you say about her iris being partially formed. So it’s still aniridia, just not as severe as some people have.

My daughter doesn’t have prescription glasses as they have said they can’t fix her vision with glasses. Is that the case for you?

I don’t wear prescription glasses, no. Doctors did try a few lenses on me when I was little, but they made no discernible difference, so I’ve never had glasses. I’ve never felt I’ve needed them either to be honest, I get on fine without. I use green-tinted sunglasses to help me with my aniridia though, which I bought from the RNIB. The green filter reduces the glare from the natural light significantly, more so than regular sunglasses do. For a child, it’s worth trying normal sunglasses to begin with, and only move on to different type of lenses if it becomes apparent that they might need them.

Is there anything in particular you struggled with as a child? Just wondering if I should get myself a really bright coat or scarf or something that could help my daughter see me.

I did have issues with bullying and not being able to see what was going on in class at my very first school, because the teachers had no idea and no training on how to help me. So that shattered my confidence. But I was moved out of there swiftly, and my confidence steadily grew in the years after that. However, it’s very important to bear in mind that was 30 years ago – training and support is much better now than it was back then. So don’t worry about that, I’m not representative of everyone else.

Ultimately, your daughter is like every other child. Every kid spends their formative years adjusting and adapting to the world around them, as they grow to learn about it and understand it, regardless of whether they have a disability or not. We all had to do it. It’s just that your daughter sees things slightly differently to some of the others, so she’ll just learn how to do things slightly differently. The fact that, like her, I was born with my condition was a good thing, I think, because this has always been normal to me. I don’t feel that I’ve lost 20/20 vision, as I never had it in the first place.

So being there to support her as and when she needs it is absolutely fine. By all means experiment to see what works – there’s no harm in trying a bright coat for instance. You’re learning about bringing up a child just as she’s learning about growing up, so you’ll be learning from each other as you go along in the months and years ahead. You’ll be surprised when you look back later at how far you’ve all come.

My daughter amazes me with certain things like catching bubbles…. would that surprise you? I’m shocked sometimes at the things she notices and other times shocked at the things she’s doesn’t.

I’m not surprised she can she them. She clearly has some level of vision, so moving things like bubbles have a fair chance of grabbing her attention. Especially if they’re big or numerous and show up well in the light. After all, being water, bubbles can be reflective of the lighting around them, so a little glint of light off one of them might be enough. She might not always spot them, sure, but sometimes she will. She’ll continue to surprise you in many ways like that I expect.

Nadine

What causes your nystagmus? Is it low vision or an underlying condition associated with the aniridia.

Hi Nadine. It’s the aniridia that caused my nystagmus, the two conditions often come hand-in-hand with each other. Not always, but it is quite common for aniridics.

My daughter was diagnosed with foveal hypoplasia. She has Aniridia, no iris and very very small cataracts. Her eye doctor said the foveal hypoplasia is what’s causing her nystagmus. Just wondering if you’ve lived with the same thing?

‘m not familiar with foveal hypoplasia myself- I’ve heard of it vaguely I think, but I don’t know anything about it – so I can’t comment on it specifically. That said, however, I’m not surprised by your doctor’s assessment, because nystagmus does often come as part of the package with a lot of eye conditions, so it makes sense.

3 thoughts on “Q&A – My Aniridia & Nystagmus”

Thanks for answering my questions.. I did a little test with my baby, bringing him from a lighted living room to a dark bedroom (not pitch black) to see if he could grab the tissues and the tissue box on the bedside table. I didn’t give him extra time for his eyes to adjust, and he was able to grab the tissues and the box.

How is it for you transitioning from lighted living room to a dark bedroom at night? How long do you think it takes to adjust to the darkness? We all need time to adjust, but I was surprised my little one was able to grab quiet quickly at the right distance.

Are there any other small fun experiments/exercises you can think of that I can try doing? thx Sam

it’s one of those things that’s very individual, so everyone’s experiences will differ. If it’s pitch black, then I won’t adjust at all. But n an average situation where there is a little bit of light to pick up on, I will usually have adjusted enough after about a minute to be able to locate the most obvious things, and within a few more minutes I will have continued to adjust a little bit more. Certainly within 5 minutes I’ll have adjusted as much as I can I think. It’s not perfect by any means, and seeing detail on things in the dark is impossible, but I can usually make out enough to work with. Although if it’s a completely unfamiliar environment, then I might be able to see objects but not know what they are.

I’m not surprised your child was able to pick up the tissues easily. They may just have relatively quick adjustment, that’s very possible. But also, it’s an environment they’re familiar with, presumably. I can quite happily walk around my house with my eyes closed, because I know where everything is. So, unless your child has never seen that bedroom before, they will already have a sense of the layout in their head. So they may have just gone straight towards the bedroom table before they could see it clearly, because they know where it is Similarly, if they know what a box of tissues looks like, then even if they can’t see the detail in the dark, they’ll be aware of the general size and shape. So that may have helped.

You can continue to do exercises like that in any case. You’re clearly learning a lot about their visual impairment from it, which is great, especially if they can see things better than you perhaps expected. It’s just about finding the balance I guess, so that the child is having fun and not finding it frustrating. You could make things progressively more challenging if you wanted, little by little, perhaps getting them to find or describe things that are smaller or in more unusual places to see how they deal with it.

The more unfamiliar or unusual the scenario or location, the more interesting their reactions will be I expect. Same goes for anything you do with them or anywhere you take them really. It depends what you feel most curious about. Whatever you decide to try with them will help you to understand them that little bit better, so it will always be a good thing.